Spinal Deformity in Russell-Silver Syndrome.

STUDY

DESIGN

Cross-sectional analysis.

OBJECTIVES

To evaluate the prevalence of scoliosis and kyphosis in patients with Russell-Silver syndrome (RSS).

SUMMARY OF BACKGROUND DATA: Russell-Silver syndrome was described by Silver and Russell in the 1950s and 1960s and is characterized by body asymmetry and other growth abnormalities. To the authors' knowledge, this is the first study to evaluate the prevalence of scoliosis and kyphosis in patients with Russell Silver Syndrome.

METHODS

The authors performed a cross-sectional analysis of 163 persons, identified through a national RSS foundation, who consented to be included in the study and responded to a general survey questionnaire. Subjects who reported a diagnosis of scoliosis and/or kyphosis were subsequently asked to submit copies of prior spinal radiographs for evaluation at the authors' center. For evaluation of scoliosis and kyphosis on the radiographs, the researchers reviewed posteroanterior and lateral standing radiographs to measure coronal and sagittal Cobb angles.

RESULTS

Of 163 respondents, 24 (14%) reported scoliosis, 5 (3.1%) reported kyphosis, and 6 (3.8%) reported both kyphosis and scoliosis, with average age of diagnosis of 8 years (range, 1-43 years). Of these respondents, 6 reported a history of bracing for scoliosis and/or kyphosis and 3 braced respondents reported having had surgery for scoliosis and/or kyphosis. An additional 3 respondents reported that corrective spinal surgery was "planned for the future."

CONCLUSIONS

Persons with RSS have a high prevalence of spinal deformity (21%) and a significant number of these patients will undergo corrective surgery (6 of 34; 18%).